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Joseph disease and Huntington disease

Protein patterns in fibroblasts and brain

Laboratory of Cellular Neurobiology (Drs. Rosenherg and Baskin and Ms. Ivy), the Department of Neurology and the Department of Pathology (Dr. Kirkpatrick), The University of Texas Health Science Center at Dallas, Dallas, TX, and the Department of Pediatrics (Ms. Bay and Dr. Nyhan), School of Medicine, The University of California, San Diego, La Jolla, CA.

Address correspondence and reprint requests to Dr. Rosenberg, Laboratory of Cellular Neurobiology, Department of Neurology, University of Texas Health Science Center at Dallas, Dallas, TX 75235.

Article abstract-Proteins were separated on two-dimensional acrylamide gels obtained from brain samples of patients with Joseph disease, Huntington disease (HD) and multiple sclerosis. Similar protein separations were made from cultured skin fibroblasts of Joseph disease patients. Two major classes of proteins, one with a MW of 50,000 probably representing the glial filamentous acidic protein, or another class with a MW of 40,000 (proteins Jc, Jd, L₁ and L₂) were increased in the cerebellum of six Joseph disease patients. The same protein species were abnormally increased in HD brains, mainly in the basal ganglia and frontal cortex. These identical classes of protein changes were present in two nosologically separate autosomal dominant neurological disorders, Joseph disease (a spinocerebellar degeneration) and HD (a basal ganglia and cerebral cortical degeneration) and may reflect a biochemical correlation of gliosis and neuronal disease. However, these changes may be evidence that the two diseases are allelic mutations of the same gene. The dominantly inherited spinocerebellar degenerations may result from a primary defecit of glial-neuronal interaction, resulting in neuronal loss but with a compensatory increase in the number of glial cells attempting to provide additional trophic-metabolic support.

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